A 46-year old man was admitted as an emergency after a collapse suggestive of syncope. In fact he had been unwell for a year with malaise and fatigue, significant weight loss, progressive breathlessness and night sweats. Indeed, he had seen his family doctor 9 months previously and was found to be polycythaemic, and had had a total of seven units of blood venesected without clinical improvement. Subsequent detailed questioning elicited a history of postural dizziness and presyncope – particularly after venesection, urinary frequency, impotence, gustatory sweating, and more recently, ascending numbness and dysaesthesia of the hands and feet, poor grip strength and bilateral foot drop.
THE EXAMINATION AND INVESTIGATIONS
On examination he looked thin and unwell. An ejection systolic cardiac murmur was noted and there was a marked postural drop in systolic blood pressure (by 40–70 mmHg at various times). There was distal weakness, generalized areflexia and glove
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